Aortic coarctation natural history, complications and prognosis: Difference between revisions

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{{Aortic coarctation}}
{{Aortic coarctation}}
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]][mailto:psingh@perfuse.org], {{CZ}}; '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]][mailto:kfeeney@perfuse.org]
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]][mailto:psingh13579@gmail.com], {{CZ}}; '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]][mailto:kfeeney@elon.edu]


==Overview==
==Overview==
An aortic coarctation is primarily symptomatic enough during childhood to be examined, approximately 80% of cases are diagnosed at this time. The remaining 20% of cases are often less symptomatic, less severe but will ultimately require correction or irreversible organ damage can occur. Common complications, when left untreated, include: rupture, infective endocarditis, congestive heart failure, and calcification of the aorta.
80% of patients are diagnosed during childhood. In the remaining 20% of cases, the symptoms are often less severe, but the coarctation will ultimately require correction in order to prevent irreversible organ damage. Common complications among patients who go untreated include: [[aortic rupture]], [[infective endocarditis]], [[congestive heart failure]], and [[calcification of the aorta]].


==Natural History==
==Natural History==
In infants with a preductal coarct, the LV output goes to the upper extremities, and the RV output goes to the lower extremities through the patent ductus.
In infants with a preductal coarct, the [[Cardiac output|LV output]] goes to the upper extremities, and the RV output goes to the lower extremities through the [[patent ductus]].
*'''Childhood:'''
*Childhood:
:*80% of cases are diagnosed in childhood.
:*80% of cases are diagnosed in childhood.
:*The preductal form is usually discovered in early infancy because it is usually severe.
:*The preductal form is usually discovered in early infancy because it is usually severe.
:*Childhood coarctation is associated with a [[ventricular septal defect]] (VSD), tubular hypoplasia of the [[aortic arch]], [[transposition]] of the great vessels, and [[mitral valve]] disease.
:*Childhood coarctation is associated with a [[ventricular septal defect]] (VSD), tubular hypoplasia of the [[aortic arch]], [[TGA|transposition of the great vessels]], and [[mitral valve disease]].
*'''Adolescence:'''
*Adolescence:
:*When first recognized in adolescents, coarctation of the aorta is generally asymptomatic.
:*When first recognized in adolescents, coarctation of the aorta is generally asymptomatic.
*'''Adulthood:'''
*Adulthood:
:*The postductal form is often less severe and discovered in adulthood.
:*The postductal form is often less severe and discovered in adulthood.
:*Infrequently coarctation of the aorta is associated with other congenital abnormalities.
:*Infrequently coarctation of the aorta is associated with other [[congenital abnormalities]].
:*In patients over the age of 30, major complications leading to death are not uncommon. <ref>Giuliani et al, Cardiology: Fundamentals and Practice, Second Edition, Mosby Year Book, Boston, 1991, pp. 1670-1676.</ref> <ref>Deal K, Wooley CF. Coarctation of  the Aorta and Pregnancy. Annals of Internal Medicine 1973, 78:706-710.</ref>
:*In patients over the age of 30, major complications leading to [[death]] are not uncommon. <ref>Giuliani et al, Cardiology: Fundamentals and Practice, Second Edition, Mosby Year Book, Boston, 1991, pp. 1670-1676.</ref><ref>Deal K, Wooley CF. Coarctation of  the Aorta and Pregnancy. Annals of Internal Medicine 1973, 78:706-710.</ref>
* 75% of patients with coarctation will have hypertension at 30 years of their age.
:*If the coarctation is left untreated, [[arterial hypertension]] may become permanent due to irreversible changes in some organs (such as the [[kidney]]).
:*If the coarctation is left untreated, [[arterial hypertension]] may become permanent due to irreversible changes in some organs (such as the [[kidney]]).


==Complications==
==Complications==
About 50% of patients with coarctation of the aorta die within the first three decades of life, and more than 75% are dead by age 50 due to:
About 50% of patients with coarctation of the aorta die within the first three decades of life, and more than 75% are dead by age 50 due to:
===Rupture of the aorta or aortic dissection===
*[[Rupture of the aorta]] or [[aortic dissection]]
*Most frequently in the third or fourth decade.
** Most frequently in the third or fourth decade.
*Dissections originate either proximally (secondary to hypertension and local stress)or distally (where the jet erodes the intima).
** Dissections originate either proximally (secondary to [[hypertension]] and local stress)or distally (where the jet erodes the intima).
*Ruptures may bleed into the esophagus, and [[hematemesis]] or [[melena]] may portend disaster.
** Ruptures may [[bleed]] into the [[esophagus]], and [[hematemesis]] or [[melena]] may portend disaster.


===Infective endocarditis or endarteritis===
* [[Infective endocarditis]] or endarteritis
*Most frequently in the second to fourth decade of life.
** Most frequently in the second to fourth decade of life.


===Rupture of the circle of Willis===
* Rupture of the [[circle of Willis]]
*Most frequently in the second or third decade of life.
** Most frequently in the second or third decade of life.
*Secondary to the increased incidence of aneurysms in this population and the presence of proximal hypertension.
** Secondary to the increased incidence of [[aneurysms]] in this population and the presence of proximal [[hypertension]].


===Congestive Heart Failure===
* [[Congestive heart failure]]
*Common in infants, often occurs with associated abnormalities such as [[VSD]] or mitral valve disease.
** Common in infants, often occurs with associated abnormalities such as [[VSD]] or [[mitral valve disease]].
*In the adult is secondary to [[hypertension]] associated with [[coronary artery disease]] or aortic valve disease.
** In the adult is secondary to [[hypertension]] associated with [[coronary artery disease]] or [[aortic valve disease]].


===Calcification aortic stenosis===
* Calcified [[aortic stenosis]]
*Result of associated [[bicuspid aortic valve]] disease that over time becomes calcified.
** Result of associated [[bicuspid aortic valve]] disease that over time becomes calcified.
 
* Premature death occurs due to
** [[Coronary artery disease]]
** [[Heart failure]]
** [[Stroke]]
** [[Aortic dissection]] or rupture
 
==Prognosis==
Prognosis is variable. The prognosis of aortic coarctation depends on whether [[balloon angioplasty]] and [[stenting]] or the [[surgery]] has been done or not.
* The mortality rate of aortic coarctation depends largely on the age of surgical repair. Left untreated, less than 20% of untreated patients live to the age of 50. However, with surgical repair, [[mortality rate]]s are far lower and survival is prolonged.
* If repaired by the age of 14, the [[mortality rate]] during the 20 years following is only 9% (a 91% survival rate).
* If repaired later than age 14, the [[mortality rate]] is 11% (a 79% survival rate).
* Among expectant mothers with a coarctation of the aorta, the [[maternal mortality]] rate ranges between 3-8%. Even with repair, there is a risk of developing complications during [[pregnancy]].


==References==
==References==
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[[Category:Congenital heart disease]]
[[Category:Congenital heart disease]]
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Latest revision as of 22:19, 14 March 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S.[2], Cafer Zorkun, M.D., Ph.D. [3]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S.[4]

Overview

80% of patients are diagnosed during childhood. In the remaining 20% of cases, the symptoms are often less severe, but the coarctation will ultimately require correction in order to prevent irreversible organ damage. Common complications among patients who go untreated include: aortic rupture, infective endocarditis, congestive heart failure, and calcification of the aorta.

Natural History

In infants with a preductal coarct, the LV output goes to the upper extremities, and the RV output goes to the lower extremities through the patent ductus.

  • Childhood:
  • Adolescence:
  • When first recognized in adolescents, coarctation of the aorta is generally asymptomatic.
  • Adulthood:
  • The postductal form is often less severe and discovered in adulthood.
  • Infrequently coarctation of the aorta is associated with other congenital abnormalities.
  • In patients over the age of 30, major complications leading to death are not uncommon. [1][2]
  • 75% of patients with coarctation will have hypertension at 30 years of their age.
  • If the coarctation is left untreated, arterial hypertension may become permanent due to irreversible changes in some organs (such as the kidney).

Complications

About 50% of patients with coarctation of the aorta die within the first three decades of life, and more than 75% are dead by age 50 due to:

  • Rupture of the circle of Willis
    • Most frequently in the second or third decade of life.
    • Secondary to the increased incidence of aneurysms in this population and the presence of proximal hypertension.

Prognosis

Prognosis is variable. The prognosis of aortic coarctation depends on whether balloon angioplasty and stenting or the surgery has been done or not.

  • The mortality rate of aortic coarctation depends largely on the age of surgical repair. Left untreated, less than 20% of untreated patients live to the age of 50. However, with surgical repair, mortality rates are far lower and survival is prolonged.
  • If repaired by the age of 14, the mortality rate during the 20 years following is only 9% (a 91% survival rate).
  • If repaired later than age 14, the mortality rate is 11% (a 79% survival rate).
  • Among expectant mothers with a coarctation of the aorta, the maternal mortality rate ranges between 3-8%. Even with repair, there is a risk of developing complications during pregnancy.

References

  1. Giuliani et al, Cardiology: Fundamentals and Practice, Second Edition, Mosby Year Book, Boston, 1991, pp. 1670-1676.
  2. Deal K, Wooley CF. Coarctation of the Aorta and Pregnancy. Annals of Internal Medicine 1973, 78:706-710.

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